Eur Radiol (2002) 12:2548–2561

DOI 10.1007/s00330-001-1286-x NEURO

Mika Okahara Anatomic variations of the cerebral arteries

Hiro Kiyosue
Hiromu Mori and their embryology: a pictorial review
Shuichi Tanoue
Michihumi Sainou
Hirohumi Nagatomi

Received: 4 July 2001
Revised: 22 October 2001
Accepted: 6 November 2001
Published online: 21 March 2002
© Springer-Verlag 2002
M. Okahara (✉) · H. Kiyosue
Department of Radiology,
Nagatomi Neurosurgical Hospital,
Omichi-machi 4–5–28,
870–0822 Oita, Japan
e-mail: okaharam@mb.infoweb.ne.jp
Tel.: +81-97-5451717
Fax: +81-97-5451745
H. Mori · S. Tanoue
Department of Radiology,
Oita Medical University,
870–0822 Oita, Japan
M. Sainou · H. Nagatomi
Department of Neurosurgery,
Nagatomi Neurosurgical Hospital,
Omichi-machi 4–5–28,
870–0822 Oita, Japan
Abstract In the embryonic period, Keywords Cerebral arteries ·
several developmental anomalies of Normal variant · MRA ·
the cerebral arteries occur. The Angiography
knowledge of these anatomic varia-
tions of the cerebral artery is impor-
tant to avoid the unnecessary surgery
and to undergo surgery or intervent-
ional radiology with safety. We re-
viewed 3000 MR angiographies and
700 cerebral angiographies of the
previous 5 years to assess cerebral
arterial system, and to illustrate the
embryological development, imag-
ing findings, occurrence, and clinical
significance of the anatomic varia-
tion of the cerebral arteries. The nor-
mal development and variations of
the cerebral arteries are depicted.
Knowledge of the anatomic varia-
tions is important since it can influ-
ence surgical and interventional pro-
cedure.

Introduction CT [2, 3, 4, 7, 8, 9]. On the contrary, small fenestrations

In the embryonic period, several developmental anomalies
of the cerebral arteries can occur. The knowledge of these
anatomic variations of the cerebral artery is important to
avoid the unnecessary surgery and to perform either neuro-
logical surgery or interventional radiology with safety.
Many anatomic variations of the cerebral artery have been
presented in conventional angiography [10, 12, 13, 14, 15,
17, 18, 20, 21, 22, 23, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34,
35, 37]. Recent developments in technology allow us to di-
agnose most of them by CT angiography or MR angiogra-
phy, or even by conventional CT and MR images. Some
variants, such as aberrant internal carotid artery or persis-
tent stapedial artery, may be recognized on high-resolution
are not detectable by even conventional digital subtraction
angiography (DSA), and are detectable by only 3D recon-
struction images from rotational DSA (3D DSA). We re-
view our cases and illustrate the embryological develop-
ment, imaging findings, occurrence, and clinical signifi-
cance of the anatomic variation of the cerebral arteries.
Internal carotid artery
Normal development
The six paired aortic arches arise from the aortic sac,
course through the visceral (branchial) arches, and termi-

Fig. 1a–c Hypogenesis of internal carotid artery. a On coronal
view of MR angiography (MRA) right internal carotid artery is
not seen. b, c High-resolution CT scan through the skull base
shows a very small right carotid canal (arrows in c). Compare
with the normal-sized left carotid canal (arrows in b)
nate in the ipsilateral dorsal aorta. The first or C1 (cervi-
cal) internal carotid artery (ICA) segment is derived pri-
marily from the fetal third aortic arches. All other ICA
segments, namely the C2–C7 (petrous to communicat-
ing) portions, represent cranial extensions of the embry-
onic dorsal aorta [1].
In the normal adult, the ICA enters the petrous bone
through the carotid canal. The vessel ascends vertically
through the carotid canal and lies anterior to cochlea
and tympanic cavity. Separation of the ICA and tym-
panic cavity is maintained by a thin bony plate (approx-
imately 0.5 mm thick). The vessel then bends anteriorly
and medially, lying inferior and posteromedially to the
eustachian tube. It finally traverses the foramen lace-
rum of the sphenoid bone to enter the middle cranial
fossa.
2549
Agenesis/hypogenesis of ICA
Agenesis of ICA (Fig. 1) is rare, with an estimated prev-
alence of 0.01% [2]. Agenesis of ICA is usually unilater-
al. Agenesis can be distinguished from acquired ICA oc-
clusion by examination of the skull base. In cases of true
agenesis, the bony carotid canal is absent or hypoplastic.
Clinically, there is an increased prevalence of associated
abnormalities, such as intracranial aneurysms, in these
cases.
Aberrant ICA
Aberrant intratympanic ICA (Fig. 2) and lateral pharyn-
geal ICA (Fig. 3) are recognized as aberrant ICA; the
former is the anomaly that occurs when the ICA takes an
aberrant course in the temporal bone and passes through
the middle ear. It is associated with the absence of the
bone plate between the carotid canal and tympanic cavi-
ty. Steffan [3] has theorized that persistence of a stapedi-
al or other anomalous artery may fix the internal carotid
artery laterally within the middle ear. Most often the
condition presents as a hypervascular mass within the
tympanic cavity on otoscopic inspection. Correct diagno-
sis is crucial lest unnecessary surgery lead to hemor-
rhage.
The lateral pharyngeal ICA is the anomalous vessel
that exhibits extreme, medial tortuosity in which the ca-
rotid artery extended to or near the midline posterior
pharyngeal wall. Embryologically, embryonic ICA un-
coils as the dorsal aortic root descends into the chest and
finally assumes a straight course in the neck. Kelly [4]
postulated that failure to fully uncoil or migrate is due to
a greater relative growth of the dorsal aortic arch result-
ing in a congenitally tortuous carotid vessel, usually
where it crosses the glossopharyngeal nerve. Clinically,
the lateral pharyngeal ICA poses a risk during both ma-
2550
Fig. 2a–c Aberrant intratympanic internal carotid artery. High-
resolution a CT scan through the skull base and b coronal recon-
struction image show left aberrant internal carotid artery in the
Fig. 3a, b Lateral pharyngeal internal carotid artery. a Axial and
b coronal CT scan demonstrate right aberrant internal carotid ar-
tery coursing along the posterior pharyngeal wall near the midline
(arrows)
jor oropharyngeal tumor resections and less extensive
procedures such as tonsillectomy, adenoidectomy, and
palatopharyngoplasty; therefore, recognition of this
anomaly and preoperative diagnosis is important.
Vidian artery and persistent stapedial artery
Normal development
At the 4- to 5-mm (26–27 days) stage of development,
the primitive mandibular artery is formed as the first aor-
tic arch regresses and the second aortic arch gives rise to
middle ear (arrows). c Frontal view of left carotid angiography
demonstrates that the left internal carotid artery lies abnormally
far laterally (arrowhead)
the hyoid artery. At the 5- to 6-mm (28–30 days) stage,
the ventral pharyngeal artery (VPA) arises as a bud from
the aortic sac, and the mandibular artery begins to invo-
lute. By the 7- to 12-mm (32–40 days) stage, the vidian
artery forms from the consolidation of the mandibular ar-
tery remnants. The hyoid artery gives rise to the stapedi-
al artery (SA), passing through the ring of the stapes. By
the 12- to 14-mm (41–43 days) stage, the external carot-
id artery (ECA) has developed from the VPA. During the
16- to 18-mm (47–48 days) stage, the SA differentiates
into a ventral or maxillomandibular and dorsal (supraor-
bital) division. The dorsal division becomes the middle
meningeal artery in the future, and also supplies the orbit
and transiently anastomoses with the ophthalmic artery.
The ECA forms its definitive branches, including the in-
ternal maxillary artery (IMA). By the 20- to 24-mm
(49–53 days) stage, the IMA links up with the maxillo-
mandibular division while the proximal SA regresses.

Fig. 4a–e The developmental stages of the stapedial artery and
vidian artery. a Following the involution of the first two aortic
arches, represented by mandibular and hyoid arteries, the terminal
end of the paired aorta of earlier stages is recognizable as the in-
ternal carotid artery (ICA) originating from the third arch. The
ventral pharyngeal artery arises as a bud from the aortic sac. b The
hyoid artery, which is above the ICA, gives rise to the stapedial ar-
tery, shown here passing through the stapes. The vidian artery
forms from consolidation of the mandibular artery remnants.
c Normal adult anatomy. The stapedial artery involutes, and the
vidian artery is assimilated into the distal maxillary artery. d Ana-
tomic configuration of typical persistent stapedial artery. e Ana-
tomic configuration of typical vidian artery. Mand. A primitive
mandibular artery; Vent. pharyng.A ventral pharyngeal artery;
Hyoid A hyoid artery; Staped. A stapedial artery; ECA external ce-
rebral artery; IMA internal maxillary artery; MMA middle menin-
geal artery. (Modified from [5])
The vidian artery is assimilated into the distal maxillary
artery within the pterygopalatine fossa (see Fig. 4) [5].
Persistent stapedial artery
A persistent stapedial artery (PSA) is a rare anomaly
(Fig. 4). The true prevalence is unknown but is approxi-
mately 1:5000 [6]. If the embryonic stapedial artery fails
2551
to involute, the PSA arises from the petrous ICA. The
PSA exists from a bony canal on the cochlear promonto-
ry and crosses the footplate of the stapes [7]. It enlarges
the tympanic facial nerve canal en route to the middle
cranial fossa, where it terminates as the middle menin-
geal artery. The foramen spinosum, which normally con-
tains the middle meningeal artery, is small or absent.
Aberrant ICAs are frequently associated with a PSA.
A PSA has been described as a cause of pulsatile tinni-
tus, and it can be associated with other middle ear anom-
alies, most commonly involving the stapes, facial nerve,
or ICA.
Vidian artery (the artery of the pterygoid canal)
The vidian artery usually arises from the ECA (Figs. 4, 5);
however, it also may originate from the petrous ICA and
pass into the pterygoid canal where it anastomoses with
a posteriorly directed branch of the maxillary artery [8].
A vidian artery can be identified on 30% of temporal
bone dissections [9]. Embryologically, if the primitive
mandibular artery regresses normally, the vidian artery
will arise from the maxillary artery; if the proximal
primitive mandibular artery persists, the vidian artery
will arise directly from the ICA.
2552

Fig. 5a, b Vidian artery.

a Frontal and b lateral view of
right carotid angiography dem-
onstrates a vidian artery arising
from the petrous ICA and
courses anteroinferiorly

Fig. 6a, b Cerebral arteries in

embryo. a Lateral view. b Ven-
tral view. BA basilar artery;
PCoA posterior communicat-
ing artery; PChA posterior cho-
roidal artery; AchA anterior
choroidal artery; Primit. Olf.
A primitive olfactory artery;
ICA internal carotid artery;
ACA anterior cerebral artery;
MCA middle cerebral artery;
PTA primitive trigeminal ar-
tery; PVOA primitive ventral
ophthalmic artery; PDOA prim-
itive dorsal ophthalmic artery.
(Modified from [5])

Clinically, it is important to evaluate this vessel in pa-
tients with intractable epistaxis. If the vidian artery is not
obliterated in maxillary artery ligations, retrograde fill-
ing of the distal maxillary and sphenopalatine arteries
may occur if the vidian artery arises from the ICA.
Vascular malformation, such as carotid–cavernous fis-
tulae or vascular tumors such as angiofibromas, para-
gangliomas, and neurofibromas, may derive their blood
supply from this vessel.
Anterior cerebral artery
Normal development
In embryos of 4–5.7 mm (28–30 days), the cranial and
caudal divisions of the ICA are established. Cranial divi-
sion of the ICA constitutes the primitive olfactory artery,
and this artery branches off the anterior choroidal artery
and middle cerebral artery (MCA). In embryos of
11.5–18 mm (41–48 days) the primitive olfactory artery
(POA) has two branches, the original one to the nasal
fossa, and the secondary one passing more medially
which represents the future continuation of the anterior
cerebral artery (ACA). At the end of this stage the latter
artery is joined with its fellow of the opposite side by the
plexiform anastomosis that turn forms the future anterior
communicating artery (ACoA). The original POA runs
into the nasal cavity, thus forming anastomosis with the
ACA. The POA dwindles [5]. There is no branch except
the POA in the horizontal portion of the ACA, and it was
postulated that the medial striate artery and the recurrent
artery of Heubner are formed from the anastomosis be-
tween the POA and ACA. A small embryonic branch
known as the median artery of the corpus callosum arises
from the ACoA and extends toward the lamina termin-
alis. This vessel normally involutes as the ACA seg-
ments distal to the ACoA develop (see Figs 6, 7).
Azygos ACA (unpaired ACA)
The unpaired arterial arrangement represents a single-
trunk arrangement that supplies both hemispheres
(Fig. 8). In this anomaly, a single trunk throughout the
ACA course is called the azygos artery. The convention-

Fig. 7a, b Anterior circulation in embryo. a Representation of an-
terior circulation in embryo. b Representation of anterior circula-
tion in normal adult. (Modified from [38])
al type is a short or long common trunk (fused). The sta-
tistical incidence of azygos ACA ranges from 0 to 5%
[10]. Padget [5] describes a vessel sited at the midline at
the 24-mm stage that she called the median artery of the
corpus callosum (MACC). Embryologically, it is sup-
posed that this variant results from fused pericallosal ar-
teries or from the persistence of the embryonic MACC
with regression of pericallosal arteries.
Azygos ACA may be observed as a finding with little
clinical significance. Azygos ACAs have also been re-
ported in association with holoprosencephaly and an in-
creased risk of aneurysm at its bifurcation [11].
The clinical importance of a correct diagnosis of
azygos ACA arises from the presence of a single trunk,
which supplies blood to both hemispheres. Its occlusion,
as a result of thromboembolic disease or erroneous sur-
gery, has disastrous effects in relation to the resulting
ischemic area.
Bi-hemispheric ACA
The bi-hemispheric pattern is constituted by a pericallosal
artery supplying the medial portions of both hemi-
2553
spheres. The opposite ACA is hypoplastic and supplies
only the callosomarginal group of branches (Fig. 9). This
variation is found in 2–7% of anatomic specimens [12].
Clinical significance is the same as the azygos ACA.
Median artery of the corpus callosum
Several terms have been used for the median artery of
the corpus callosum (MACC), including medial ACA,
median callosal artery, superior callosal artery, third A2
artery, triplicated ACA, and accessory ACA (Fig. 10).
As a branch of the ACoA, the MACC runs parallel to
and behind the normal pericallosal artery. The MACC is
known to supply blood to the corpus callosum, septal
nuclei, septum pellucidum, rostral portions of the fornix,
and a portion of the frontal lobes. A 64% incidence in fe-
tal and neonatal autopsy material and a 3.2–22.0% inci-
dence in adult autopsy material have been reported [13].
Embryologically, it is postulated that this variant results
from persistence of embryonic MACC or marked devel-
opment of MACC because of hypoplastic ACA, but the
real cause of this anomaly is unknown.
Of particular clinical importance is the frequently en-
countered MACC, which becomes one of the draining arte-
ries of the ACoA aneurysm. It has been reported that it can
be difficult to identify such aneurysms, depending on their
orientation, and it is easy to damage them during surgery.
Fenestration of ACA and ACoA
In previous reports, the incidence of fenestration of the
ACA (Fig. 11) was 0.1–7.2% in autopsies. In many cases
fenestration occurred at the horizontal segment of ACA,
and it has been reported that the incidence of fenestration
of the ACoA (Fig. 12) was 7.5–40% in autopsies [14].
The real cause of these anomalies is obscure, but it is
postulated that these anomalies are the remnants of the
plexiform anastomosis. Clinically, a fenestration of
ACoA and a small fenestration of ACA might be mis-
taken for ACoA aneurysm on MRA. Diagnosis of those
cases is difficult or impossible using conventional DSA.
Persistent primitive olfactory artery
The persistent primitive olfactory artery (PPOA) de-
scribed above is characteristic in that it arises from the
terminal portion of the ICA, runs along the olfactory
bulb, makes an abrupt posterior turn behind the olfactory
bulb, and finally becomes the distal ACA (Fig. 13). It is
associated with the absence of ACoA and the recurrent
artery of Heubner [15]. This anomaly may be overlooked
because of its rarity, but a high incidence of cerebral an-
eurysms should be kept in mind in clinical situations.
2554

Fig. 8a–d Unpaired anterior

cerebral artery. a Magnetic res-
onance angiography demon-
strates a single trunk (azygos
artery) originates from the con-
fluence of both A1 tracts. b In
another case, frontal view of
right carotid angiography and
c 3D reconstruction image
demonstrate unpaired anterior
cerebral artery with small aneu-
rysms (arrows). d Representa-
tion of Azygos artery

(47–48 days) stage, the MCA has become more promi-

nent and supplies branches that spread over the cerebral
hemisphere [5]. The MCA development is intimately re-
lated to development of the sylvian fissure and insula.
Before the insula and sylvian fissure form, cortical
branches of the MCA ramify directly over the lateral sur-
faces of the cerebral hemisphere. As the frontal, parietal,
and temporal lobes are formed and lateral fissure begins
to develop, the MCA branches follow the deepening de-
pression that will form the insula. At birth, the insula is
completely buried within the sylvian fissure and the
MCA has essentially attained an adult configuration
[16].

Fig. 9 Bi-hemispheric anterior cerebral artery. Representation of

Bi-hemispheric anterior cerebral artery Duplicated and accessory MCA

In 1973 Teal et al. [17] proposed using the term “dupli-

Middle cerebral artery
Normal development
In embryos of 7–12 mm (32–40 days), the middle cere-
bral artery (MCA) derives from the primitive internal ce-
rebral artery proximal to the ACA. By the 16- to 18-mm
cation of MCA” (Fig. 14) to characterized the two ves-
sels originating from the distal end of the ICA, and the
term “accessory MCA” (Fig. 15) to describe the anoma-
lous vessel originating from ACA, that branch supplying
the cortex in the distribution of the MCA. Komiyama et
al. [18] has reported that duplication of the MCA sup-
plies the anterior temporal lobe, and that the accessory

Fig. 10a–c Median artery of
the corpus callosum. A 3D re-
construction of a left carotid
angiography and b MRA dem-
onstrate the median artery of
the corpus callosum (arrows)
which originates from anterior
communicating artery. c Repre-
sentation of the median artery
of the corpus callosum
MCA supplies the anterior frontal lobe. The incidence of
accessory MCA in autopsies is reported to be 0.3–2.7%,
and that of duplicated MCA 0.7–2.9% [19, 20].
However, the embryological explanation of the pres-
ence of anomalies and variation of MCA is not clear, it
has been postulated that accessory MCA or duplicated
MCA is persistent anastomoses between the ACA and
MCA [21].
Knowledge of anomalous early ramification of the
MCA is important in the surgical dissection of cerebral
aneurysms and in understanding the collateral blood sup-
ply in ischemic stroke associated with duplicated or ac-
cessory MCAs.
Carotid–basivertebral anastomosis
Normal development
Embryonic cervical carotid–vertebral anastomoses (Fig. 16)
are composed of eight pairs of intersegmental arteries.
The most caudal intersegmental artery is called the prim-
itive proatlantal artery or suboccipital artery. The verte-
bral arteries are formed as plexiform longitudinal anasto-
2555
moses between the six cervical intersegmental arteries
(C1–C6). In the 5- to 6-mm (28–30 days) embryo, these
vessels provide the proximal supply to the longitudinal
neural arteries via the primitive proatlantal arteries and
the cervical carotid–vertebral anastomoses. By involu-
tion of the ventral segments of these arteries, the verte-
bral artery (VA) loses connection with the primitive
arches of the aorta and the carotid artery. Only the ven-
tral part of one proximal intersegmental artery persists
and makes a connection to the subclavian artery. The
seventh cervical segments (C7) are enlarged and will be-
come the subclavian arteries.
In the 5- to 9-mm (31–36 days) embryo the longitudi-
nal neural arteries fuse across the midline to definitive
basilar artery (BA).
Anastomotic connections between the developing in-
ternal carotid and basilar arterial systems exist in early
fetal life, at a time when embryonic length is 4–5 mm.
These vessels are named after the cranial nerves with
which they course, and include the trigeminal artery, otic
artery, and hypoglossal artery. The proatlantal interseg-
mental artery courses suboccipitally to form an anasto-
mosis between the carotid and vertebral artery, and
therefore is not considered a true carotid–basilar commu-
2556

nication. After formation of the posterior communication
artery from the caudal branch of the ICA, the preseg-
mental arteries are normally obliterated, starting with the
otic artery, followed in turn by the hypoglossal and tri-
geminal arteries [22].
Persistent primitive trigeminal artery
The persistent primitive trigeminal artery (PPTA;
Fig. 17) is the most frequent of these embryonic con-
nections and has been observed in 0.1–1.0% of angio-
grams and autopsies [23]. The presence of the PPTA in
adults mirrors the 11- to 14-mm (41–43 days) embryon-
ic stage [5]. A PPTA arises from the cavernous ICA
near the posterior genu, and may follow either a para-
or an intrasellar course. The PPTA supplies blood to
both posterior cerebral arteries and superior cerebellar
arteries via the distal BA, and when it persists there is
no flow-related stimulus for the BA proximal to the
anastomosis to develop along with the embryo. Boyko
et al. [24] has considered that this observation explains
the frequent association of BA hypoplasia with PPTA.
Ohshiro et al. has classified this into two types: a medi-
al type in which the artery runs through the dorsum
sellae and perforates the dura mater near the clivus, and
a lateral type in which the artery runs between the sen-
sory root of the trigeminal nerve and the lateral side of
the sellae and penetrates the dura mater medial to
Meckel’s cave [25]. Recognition of the PPTA can be
important in surgical procedures in the cavernous sinus
or the posterior fossa, and may prevent injury or dis-
ruption of the PPTA. Endovascular procedures should
be modified accordingly to avoid ischemia to the brain
stem and the cerebellum. The PPTA are also associated
with increased prevalence of other vascular abnormali-
ties such as aneurysms. Aneurysms are found in nearly
14% of all cases [26].

Persistent primitive trigeminal artery variant

The persistent primitive trigeminal artery variant

Fig. 11a, b Fenestration of anterior cerebral artery. a Representa-
tion of the fenestration of the anterior cerebral artery and anterior
communicating artery. b Magnetic resonance angiography demon-
strates fenestration (arrows) in the horizontal segment of anterior
cerebral artery
(PPTAV; Fig. 18) has been reported as a subtype of
PPTA. The vessel is the anatomic connection between
primitive trigeminal artery, which should be connected
with the BA, and one cerebellar artery (superior cerebel-

Fig. 12a, b Fenestration of an-

terior communicating artery
(ACoA). a Three-dimensional
digital subtraction angiography
(DSA) image of the left carotid
angiography demonstrate fen-
estration (arrows) in ACoA.
b In another case, 3D DSA im-
age of the left carotid angiogra-
phy shows fenestration (arrow-
heads) of ACoA with an aneu-
rysm (arrow)

Fig. 13a–c Persistent primitive olfactory artery. a Oblique view
of MRA shows the anomalous artery (arrow) originating at the bi-
furcation of the left ICA. It courses anteromedially and makes a
hairpin turn to the posteriorly. b Axial T2-weighted MRI shows
anomalous flow void originating left ICA and running anteromedi-
ally (arrowheads). c Representation of the persistent primitive ol-
factory artery
lar artery or anterior inferior cerebellar artery or posteri-
or anterior inferior cerebellar artery). Embryologically, it
is supposed that a type of the PPTA associates with par-
tial remnant of ipsilateral posterior longitudinal neural
artery.
2557
Fig. 14a, b Duplication of middle cerebral artery. a Representa-
tion of the accessory and duplicated middle cerebral artery.
b Magnetic resonance angiography demonstrates left duplicated
middle cerebral artery (arrows), which originates from left ICA
Persistent primitive hypoglossal artery (PPHA)
The persistent primitive hypoglossal artery (PPHA;
Fig. 19) is the second most common carotid–basilar
anastomosis and has been observed in 0.1–0.25% [27].
This vessel arises from the cervical ICA, usually at the
C1–C2 level, and curves posteromedially toward an en-
larged hypoglossal canal. Brismar [28] proposed that the
following two criteria be fulfilled for diagnosis of PHA:
The PPHA should leave the ICA as an extracranial
branch; and it should pass through the hypoglossal canal
before joining the caudal part of the BA. De Caro et al.
[27] suggested that PPHA may be associated with an
anomalous structure of the vessel wall and exposes the
basilar trunk to an unusual hemodynamic stress, predis-
posing to the onset of aneurysms with possible hemor-
rhagic consequences.
2558
Fig. 15a, b Accessory middle cerebral artery. a Magnetic reso-
nance angiography and b frontal view of left carotid angiography
demonstrate left accessory MCA, which originates from the proxi-
mal portion of the left anterior cerebral artery. The accessory
MCA has perforating artery
Fig. 16 Normal development of carotid–basivertebral system.
PCoA posterior communicating artery; PTA primitive trigeminal
artery; POA primitive otic artery; PHA primitive hypoglossal ar-
tery; PAA primitive proatlantal artery; CISA cervical intersegmen-
tal artery; VA vertebral artery. (Modified from [5])
Proatlantal artery, vertebral artery,
and basilar artery
Normal development
Normal development of the vertebral artery (VA) and
BA is described in “Carotid-basilar anastomosis” (see
Fig. 16).
Proatlantal artery
It is postulated that the type-1 proatlantal artery is a per-
sistent primitive proatlantal artery, and the type-2 proat-
lantal artery is a persistent primitive first cervical inter-
segmental artery. The type-1 proatlantal artery rises from
the ICA or ECA and runs upward and dorsolaterally, as-
cending to the occipitoatlantal space without passing
through the transverse foramen of any cervical vertebra.
It joins the fourth segment of the VA. The type-1 proat-
lantal artery courses horizontally above the atlas to enter
the skull through the foramen magnum, giving off ipsi-
lateral vertebral artery. The type-2 proatlantal artery rises
from the ECA and joins the third segment of the VA be-
low the first cervical vertebra [29].
Fenestration or duplication of vertebral artery
The vertebral artery (VA) fenestration (Fig. 20) is found
in 0.23–2.0 % [30, 31] of autopsies or angiographic stud-
ies. Angiographically shown duplications of the origin of
the VA are very rare. Embryologically, the extracranial
fenestrations are most likely caused by the persistence of
cervical intersegmental arteries, whereas the intracranial
components probably arise from persistent basivertebral
anastomoses [32].
The VA fenestration is often associated with other
anomalies of the brain, spinal cord, and spine; these in-
clude fused vertebrae, other vascular anomalies, and an
increased prevalence of aneurysms and vascular malfor-
mations [33]. Uchino et al. [34] have reported the fre-
quency of coexistence of arteriovenous malformation
and VA fenestration was 7%.
Fenestration of basilar artery
Basilar artery fenestration (Fig. 21) is reported in 1–5%
of autopsies and in 0.1–1.9% of angiographic series
 2559

Fig. 17a–d Persistent primitive trigeminal artery. a Axial T2-

weighted image demonstrates the right-sided flow void of the tri-
geminal artery (arrow). b Coronal views of MRA demonstrate
anomalous carotid–basilar anastomosis (persistent primitive tri-
geminal artery; arrow). c Frontal and d lateral views of right ca-
rotid angiography show filling of the basilar trunk through the per-
sistent primitive trigeminal artery (arrow)

Fig. 19 Persistent primitive hypoglossal artery. Lateral views of

left carotid angiography demonstrate persistent primitive hypo-
glossal artery (arrow), which originates from cervical portion of
the internal carotid artery and anastomoses basilar artery through
the hypoglossal canal. Small aneurysms of the persistent primitive
hypoglossal artery are also noted (arrowheads)

Fig. 18 Persistent primitive trigeminal artery variant. Lateral view

of left carotid angiography demonstrates an anomalous carotid–
superior cerebellar anastomosis (arrow)
2560
Fig. 20 Fenestration of vertebral artery. Frontal view of right ver-
tebral angiography demonstrates fenestration in the right vertebral
artery (arrows)
Fig. 21 Fenestration of basilar artery. A 3D reconstruction image
demonstrates fenestration in proximal basilar artery (arrows). An
aneurysm of the basilar tip is also noted (arrowhead)
[31]. It can occur anywhere along the course of the BA
but is most frequent in the proximal basilar trunk, close
to the vertebral arteries [35]. Although the BA is formed
by the fusion of two longitudinal neural arteries, an in-
complete fusion may lead to a fenestration. The high in-
cidence of aneurysm in association with BA fenestration
has been reported. Black and Ansbacher [36], in a
pathological study, described defects in the vessel wall
of each end of fenestration, which may promote growth
of an aneurysm, with hemodynamic forces and structur-
al degenerative changes in the vessel. Tasker and Byrne
[35] have suggested that aneurysms associated with a
fenestration tend to have broader necks than those of a
comparable size at other sites, and may therefore be
more difficult to treat successfully via the endovascular
route.
Fig. 22 Fetal posterior cerebral artery. Axial MRA shows the
right fetal posterior cerebral artery (arrows). Left persistent primi-
tive trigeminal artery is also noted (arrowheads)
Posterior cerebral artery
In embryos of 4–5.7 mm (28–30 days) the cranial and cau-
dal divisions of the internal carotid artery are established.
The caudal divisions anastomose with the cranial ends of
the longitudinal neural arteries. The caudal divisions then
regress and become posterior communicating arteries
(PCoAs). The caudal divisions also supply the stems of the
posterior cerebral arteries (PCAs). By the 40-mm (8-week)
stage, the PCAs can be identified as the posterior commu-
nication of the PCoAs. The PCoAs normally regress in cal-
iber as the vertebrobasilar system develops [5].
Fetal PCA
If the embryonic PCoA fails to regress, the dominant
blood supply to the occipital lobes comes from the ICA
via the fetal PCA instead of from the vertebrobasilar
system (Fig. 22) [37]. This occurs in approximately
20–30% of cases [38]. Clinically, if we treat the
ICA–PCoA aneurysm, we should not occlude fetal PCA
to avoid infarction of the PCA territory.
Miscellaneous
Duplications and fenestrations of the PCA and superior
cerebellar artery (SCA) occur as well as in other cerebral
arteries. Common trunk of the PCA and SCA is seen in
2–22% of cases. These variations are important in the
surgery and endovascular treatment of the BA aneurysm.
In conclusion, knowledge of anatomic variations of
the cerebral arteries is important since it can influence
with surgical and interventional procedure.
Acknowledgements We thank N. Akada and D. Uchida for the
photographic work.

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